It Happened That Night

Hey guys! So once again, I am writing this while inpatient at Children's Hospital Boston. I am currently here due to another cellulitis infection which initially began Wednesday evening. It was around 9pm that I awoke to a tremendous headache, scorching pain in the left buttock of my KTS area, a fever and a slew of other worrisome symptoms. As many of you who deal with these infections know, the onset of one is hardly pleasant. Since my pain was not yet out of control, I talked to my mom and dad and we decided to check in about how I was doing in about half an hour or so. I think we all knew that it was only a matter of minutes before I would be on my way to the ER, though. Still, I fought to remain optimistic in my mind while all of this was going on. I tried to convince myself that the pain may not get worse, and that I could perhaps hang on until tomorrow when my primary doctors were in and I would not have to go through the whole ER ordeal. However, the pain got substantially worse over the next half hour or so and suddenly it felt as though various steak knives were being jammed into my left buttock – it's a pain so intense, so deep, that I shudder when merely thinking about it. At that point, the hurting was unbearable and my mom and I were out the door to the ER in no time.

Emotionally, this was a rather hard experience as well because I had been taking Keflex twice daily at 2000 mg (a preventive dose Infectious Diseases put me on after the severity of my last infection). Once again, my body was breaking through yet another medicine and it was not only disheartening, but scary , too.

Perhaps this is the real kicker, though: For the past three weeks leading up to that infection, I had not been able to walk on my left leg due to severe pain that started at my frontal thigh area and stretched down past my knee cap. Throughout this time period I was in contact with Dr. Trenor and Dr. Fishman, but Dr. Alomari was out of town and I knew he would be a key component in getting to the root cause of what was causing me not to walk. So, just earlier that day (Wednesday), I had been at Children's and had a somewhat lengthy doctor's appointment with Dr. Alomari (who had just returned from his time abroad that day) in regards to this new and very problematic area. He ultrasounded my leg, and within minutes was able to diagnose me with “acute thrombophlebitis.” To treat it, I would have surgery on Monday, February 24^th in interventional radiology with Dr. Alomari. I came home from the appointment incredibly fatigued, so off to sleep I went. Little did I know my body had a bit of a surprise in store for me just a couple of hours later...

So, as I rode to the ER the night of the infection, I was not only dealing with the sensation of stake knives going into my butt but the pain from the widespread thrombophlebitis as well. Between not being able to walk nor sit, making it downstairs to my car proved to be quite an obstacle in itself.

Anyway, I am now here being treated with three IV antibiotics and go for surgery tomorrow with Dr. Alomari. The teams are doing a fantastic job of providing me with great pain relief, and everyone is so incredibly friendly! While the redness in my buttock has certainly gone down, it is still rather uncomfortable which tells me this infection will not be a quick one to treat.

I will update more soon! If you have any questions, please feel free to leave a comment below and I will be glad to answer it.

Below are recently taken pictures of the area with Thrombophlebitis.

I got to spend some time with my younger sister tonight too which was so incredibly awesome! I love her to pieces. Here is a picture of us from a mere few hours ago.

I will be in touch very shortly! Wishing you all well. - A

Relentless Chronic Pain, Establishing Normalcy

“Walking through the city street, is it by mistake of design, I feel so alone on a Friday night can you make it feel like home if I tell you your mine, It's like I told you Hunny”

I hate the feeling of a flare-up, it drives me incredibly wild to be absolutely honest! Perhaps the essence of the problem is that one cannot always be sure it is merely a flare-up in that moment. One day about a week ago, I awoke with a cellulitic red spot located somewhere between my foot and ankle. It did not look bewildering by any means, but certainly suspicious. The other evening, I had to awaken my mom in the midst of her slumber to dip into my emergency stash of oxycodone as the pain was radiating throughout the new red spot and the entirety of my lower leg. This pain was barbarous; it would not allow me to sleep and was certainly an anomaly as far as my everyday painful sensations are concerned. It could have been inflamed veins, although I will likely never know the real source with such an inconspicuous condition (unless I had chosen to venture to the emergency room). Fortunately for me, no further symptoms accompanied this area such as temperatures or chills and what not. So, the pain medicine eventually kicked in and the next day the area in its entirety felt much better (a heating pad accompanied the medicine and did great justice as well, I believe). However, the spot still remains although I am still very much so stable in vitals and other telling symptoms.

I am continuing on my IC Cephalexin 500 MG capsules twice per day and I truly feel they are doing the heavy lifting in maintaining my health. I will be on them for at least three more weeks or so. While they can be rather upsetting to the stomach, it is far worth the residual side effects to be home and in a state of “normalcy.” Discovering normalcy, however, is still a startling challenge living with a chronic condition that causes me so much relentless chronic pain. The infections are gone (at least for now) and I do not mean to belittle that greatness by any means. However, the lower leg pain is so incredibly persistent and vicious in nature that at days I am very much so overly-sensitive and on edge; to say I am emotionally well-adjusted at present would be somewhat of a lie. However, I am doing my best to maintain honest, healthy friendships and venture out every now and then despite the pain. Sometimes, I find myself lashing out at those around me and being incredibly irritable; this is by no means the way in which I like to conduct myself. I feel guilty quite often about this, and try to communicate my remorse to those in which I may have unintentionally hurt. However, I cannot use my pain as an excuse to treat people poorly or I will be doing so for the rest of my life! By no means do I want my legacy to be that of a moody Mindy or bitter Betty.

Friends urge me to be optimistic about future breakthroughs in the medical world, but I have great trouble doing so. I understand where they are coming from, but they simply cannot understand my point of view unless they are the ones in pain 24/7. It eats at one's nerves, and chronic pain has been scientifically proven to mess with the brain's chemistry. Perhaps, then, it is no wonder I often find myself anxious and depressed despite being under the care of a talk therapist and doctor. However, I am greatly anticipating my appointment on January 3^rd to meet with my doctors to address issues of what we can make better with my Klippel.

Below is a picture of me with my sisters on Thanksgiving. It was a truly lovely day as I got to celebrate with the people who have been there for me through the worst of times; at my ugliest, sickest, and meanest. Yet, I know they are not going anywhere and I know I am indeed very blessed to blanketed in such unconditional love. May you all be well. - A  

Continued Antibiotics, etc.

Hey everyone! I know I have not written in a bit, much to my deepest regret. Access to a fully functioning computer still proves to be challenging at this point, but I will have a new one by Christmas!

I have been out of the hospital since the end of September now and it has been such a delightful treat. Just earlier today, I spoke with one of the VAC nurses Erin in regards to my antibiotic situation. Initially, upon coming home, I was taking 2000 mg of cephalexin daily (twice a day). We then lowered it to 1000 mg of cephalexin daily and I was due to finish it today. However, due to my unchallenged success on these oral dosages, my doctors have made the decision to keep me on it for another two months as a prophylactic. I was quite pleased with this decision as I have my sister's wedding towards the end of December. The medicine does make me feel slightly sick, but probiotics can help counter this.

As far as debulking my ankle is concerned, I am planning to move forward with this procedure. However, nothing will be finalized until I meet with Dr. Spencer, Dr. Alomari, and Dr. Fishman on January 3^rd. 

I am still dealing with a great deal of anxiety and depression, however am working very hard to make it through each day and to be at least somewhat productive. The hardest part of being out of the hospital is readjusting to at home life while trying to maintain my health and reestablish normal daily habits.

Hope to update soon!

- Arianna

Round and Around and Around We Go...

Seems these are rather tumultuous times for me and a multitude of my Klippel sweethearts. Many of them are inpatient at present; as of today, I am too. Once again, I am fatigued emotionally and physically. As for what I am being treated for, I cannot say with total certainty at this point in time. We have yet to rule out infection/deep clot/both. The good thing is I was able to catch it before I got systemic...which very well may be attributed to the 26 day intensive antibiotic regimen I have been on.

In other words, that may be masking things from getting out of control.

In my last post, “last evening”, I chronicled my initial flare-up that led to this hospitalization. While the overt systemic systems died down at rapid speed, I experienced yet another problem this morning. I awoke around 1 in the morning to a throbbing in my thigh that felt cellulitic (based on previous infections). Several hours later and the pain persisted. Between the butt and the thigh both being in abnormal pain, I felt it adamant to be seen and now I am here yet again. There is no striking redness, although there is a feint tint of some.

While I am not one to ever speak poorly of the beloved hospital that has saved my life dearly so many times, I must say I was not impressed with a couple of the vascular anomalies staff members today. I will not mention names, nor go into all of the specifics, but I was talked down to quite a bit (as was my mother) and even toyed with mentally to a degree. I am not referring to the specialists themselves by any means, but rather a couple of the people who work beneath them. Both who mistreated me (especially knowing the complexity of my situation these past 6 or 7 months) lacked empathy and compassion entirely. I just have to remember that I am indeed here for the specialists who are so expert at their crafts.

For now, pain medicine awaits along with bedtime. It was another 8 hour seemingly endless day in the ER prior to being placed into a room.

As always, love to you all - A

In A Moment's Notice (Part 1)

When I last wrote, things were seemingly getting better; albeit slowly. Still, everything was being managed just fine and the infection appeared to be responding well to intravenous treatment of antibiotic. Then, I woke up (while still inpatient) with the chills; it was previously planned I would go home that day... prior to the events that were unfolding at a moment's notice. Suddenly, without even the slightest of warning, I was wandering the vast mountains of Antarctica in nothing but a tank top and shorts. There were not enough blankets in the world to contain the numbing cold that occupied every crevice of my body. My temperature shot up to just under 104. It seemed the infection had progressed, and there was certainly no question now that it had entered my bloodstream. The usual people were involved in treating me, including Dr. Fishman, Dr. Alomari, infectious diseases and the allergists.

The next few days were utter hell as we fought to control the infection. I couldn't get out of bed to use the bathroom; when I stood up, it felt as though I was in a wind-tunnel. Standing would simply not suffice, for I could not maintain balance of any sort. Bed pans were a staple throughout this time period. Even getting my shivering body up on the bed pan was a challenge in itself, especially given where the infection was located (my back thigh). My energy was tossed completely, and lifting my head was something that seemed to be a tremendous feat.

The only time I would start to feel any relief was when the oxicodone, morphine,and moltrin were administered and started to kick in. Eventually, though, my fever would progress back to its just under 104 state, and the unrelenting chills would return along with the dizziness, nausea, intense headache, all over achi-ness, etc. Not to mention the thigh infection itself... My body wasn't done surprising me yet, however. I woke up the following day with two huge clots, one in each arm. They as well were infected. I wish I was joking. No doctor had ever seen anything like it before and are still having difficulty grasping the concept of how this may have occurred.

When people see my leg, they think it is just my leg that is impacted by this lovely condition we call Klippel-Trenaunay Syndrome. Fair enough, but for those of us who know better, we know that is far from the truth. We know it can get into our bloodstream and cause sepsis, travel to other realms of the body and wreak utter havoc. We know better, unfortunately.

I returned home from the hospital yesterday afternoon and will be writing a lot more about my time during my ten day stay. My KTS family as always pulled through for me and were the most incredible support group I could have asked for. Never could I have imagined people with such endearing hearts and souls being there for me day and night, that which I am incredibly grateful for. Think of this as somewhat of an introductory post.

My KT loves, whether you are a family member of a patient or a patient yourself, may you be at peace right now. If you are not, may your pain soon subside. May you know you are not alone and have a bundle of people in your corner at all times, no matter how isolated you may feel from the outside world at any given moment.

Love always,

Arianna

Physical Therapy

Hey everyone. So there has been no improvement in my foot yet; therefore, it has been determined the clyndamycin antibiotic is not working. In the meantime, I am being kept comfortable with a heavy dosage of pain medicine. Today, I started physical therapy. Even with the pain medicine in effect, I am having a hard time walking on my foot and therefore I need to be taught effective methods for maneuvering my way around until the foot starts to heal. The physical therapist came in and basically just taught me how to use the walker from my bed to the bathroom. It is extremely simple; just move the walker, take a step with your left foot, and then your right one follows. Below is a picture of me in progress with the physical therapist. - A

Back in Hospital

Hey guys - I am back at Children's Hospital Boston due to another cellulitis infection. This one is primarily in my foot region. Pain wise, it has been one of the worst ones I've had in a while. As far as systemic symptoms are concerned, I have been experiencing nausea, tiredness, overall fatigue and low-grade temperatures but nothing striking. For me, each infection has something that stands out about it. For example, with some infections it is the incredibly fierce crayola redness that imparts upon the infected area. For others, it is how sick I got systemically. For this one, it is the relentless pain that is rampant throughout the foot and ankle area. I've been here a day now, and I still cannot bear any weight on it.  It is swollen and red, although the redness is not especially vibrant by any means.

I am being treated on IV clyndamycin and am being given morphine for pain. One thing in which I have learned, however, is that IV pain medications do not last as long as the oral ones (or so I have been told). Therefore, given the intensity of the pain in which I am experiencing, they may switch me over to oral morphine.

Above is a picture from me in the ER last night. As usual, all of the nurses have been so incredibly friendly and helpful. One of my nurses, Christina from 10 NW, is so sweet and friendly to talk to! I had her last during my last hospital trip as well. A good nurse can undoubtedly make a huge impact - so never forget to smile and say thank you! : ) - A

Update 2 on Sepsis

This infection has taken so much out of me; when I stand, I feel like I need to sit down because I am so fatigued. I am incredibly thirsty all of the time so I am constantly drinking fluids. Aside from that, the area is still somewhat sore. I still have ways to go before reaching my baseline, but I am so much better than I initially was in the hospital, so I'm grateful for that.

I've come a long way since the hospital

I still have to monitor my temperature a lot despite being on Avelox 400 mg daily (for a grand total of three weeks). The reason for this is to make sure I am not breaking through the medicine. So far I have remained lucky and my temperature has remained at baseline.

I woke up today to find out about the horrific tragedy that happened approximately 30 minutes from my house, the Boston Marathon bombings. To all injured and impacted, my deepest condolences. Boston is a strong city with the best hospitals, so at least the injured were close to the best care possible. Still, I cannot even imagine having been impacted by such a horrible ordeal. May we all keep the victims in our thoughts and prayers.

See You Later, Boston

I'm going away to Atlantic City for three days with my grandparents! Even though I have to bring a lot of homework and studying with me, it will feel good to escape my familiar surroundings for a little bit. I brought some Avelox in case an infection pops up, although I know the chances of that are very slim. Still, got to be prepared!

Have a lovely weekend,

A

Hi everyone,
I got so much positive feedback on my last post and wanted to express my sincere gratitude. It's such a priveledge to live in an age in which I have this kind of platform in order to raise awareness for Klippel-Trenaunay Syndrome. Social media is truly such a blessing, especially for us KT Patients since there are so few of us in the world. For those of you who don't know, we have literally been named one of the rarest diseases by some medical outlets (Yes, we are an extremely rare breed!).

I am taking this time to continue to recover from my cellulitis infection before I go back to college full-time. My infection is getting better as opposed to worse so I am very fortunate the 400 mg/daily of Avelox has worked wonders yet again! I hope you all are well! - A

So the redness of the initial infection spot (the thigh) has spread its way up to the left side (KTS inpacted) buttock. The redness is the same of all my typical cellulitis infections, but there is no tenderness there as of right now.

Systematically, I still have a low grade fever and just feel tired/weak. However, that is to be expected with these kind of ordeals.

For now, I just continue on oral antibiotics unless things take a sharp turn for the worst.

So, some of you may be wondering, why do some (of my) infections require hospitalization while others do not? I will try my best to make this as simplistic as possible.
- presence of acute systematic symptoms: I have had fevers as high as 105 when I have gotten these cellulitis infections; anything over 101 typically insinuates I need the emergency room. Some systematic symptoms (temperature aside) include vomiting, extreme dizziness, intolerable pain in infected area, inability to stand, sweating profusely, having the chills, massive headaches, being dehydrated, etc.. Those are some of the main ones that have come to my mind.

Fortunately, I have not been that kind of sick since June. With this one, I just have a little headache and, once again, a low grade fever. I am incredibly sore but it does not feel like I have knives being jabbed into my thigh (and thank god for that).

I am also in extremely close proximity to the hospital. If things do go sour, it is approximately a half hour from here to the emergency room.

I am lucky, and as always, will heal.
I start school in ten days, however, so hopefully it will be sooner rather than later! - A

I am healing so incredibly well - one thing in which I have always said about my body is that it is quick to get sick but always responds to treatment in a rapid manner; the sight of the infection looks substantially better. That's the thing with KTS  though - everything can look amazing on the outside while there is still a momentous amount of trouble lurking beneath the surface. This is why it is vital to always finish your medication down to the very last pill - you'd be surprised how quickly the seemingly gone infection can appear yet again (I have learned this the hard way).

A couple of pictures from the holidays:

Forget about all of the presents - this is the stuff that truly matters in life.

- A